Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of Dyke-Davidoff-Masson syndrome in Korea

국소적 경련과 편마비를 동반한 Dyke-Davidoff-Masson 증후군 1례

  • Lee, Jun Hwa (Department of Pediatrics, Catholic University of Daegu, School of Medicine) ;
  • Lee, Zee Ihn (Department of Physical Medicine & Rehibilitation, Catholic University of Daegu, School of Medicine) ;
  • Kim, Ho Kyun (Department of Radiology, Catholic University of Daegu, School of Medicine) ;
  • Kwon, Soon Hak (Department of Pediatrics, Kyungpook National University School of Medicine)
  • 이준화 (대구가톨릭대학교 의과대학 소아과학교실) ;
  • 이지인 (대구가톨릭대학교 의과대학 재활의학교실) ;
  • 김호균 (대구가톨릭대학교 의과대학 방사선과학교실) ;
  • 권순학 (경북대학교 의과대학 소아과학교실)
  • Received : 2005.08.16
  • Accepted : 2005.10.24
  • Published : 2006.02.15
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Abstract

Dyke-Davidoff-Masson Syndrome (DDMS) is a rare condition characterized by asymmetry of cerebral hemispheric growth with atrophy on one side, ipsilateral compensatory osseous hypertrophy, and contralateral hemiparesis. We experienced a 17 month-old male who presented with left focal clonic or tonic-clonic seizures accompanied by left hemiparesis and developmental delay. Brain MRIs demonstrated progressive atrophy of the right cerebral hemisphere with dilatation of the lateral ventricle, expansion of the ipsilateral frontal sinus with calvarial thickening, and elevation of the petrous pyramid and orbital roof. Brain SPECT showed a decreased volume of the right hemisphere with reduced blood flow. We therefore report a case of DDMS with a review of the literature.

Dyke-Davidoff-Masson 증후군은 일측성 대뇌 반구의 위축과 동측의 대상성 골비대, 반대측의 편마비 등을 특징으로 하는 드문 신경질환이다. 본 증례는 17개월 남아가 좌측 국소적 경련외에 좌측 편마비 및 기타 발달지연을 동반한 경우이다. 당시 시행한 뇌 자기공명영상에서 우측 대뇌 반구의 진행성 위축과 뇌실 확장, 두꺼워진 두개관과 동측 전두부 부비동의 확장, 추체 접형골의 거상을 보였고 뇌 SPECT 촬영상에 우측 대뇌 반구의 위축 및 혈류감소를 보였다. 이 같은 소견들은 Dyke-Davidoff-Masson 증후군에 합당하여 이에 저자들은 본 증례를 문헌 고찰과 함께 보고한다.

Keywords

References

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